Hydrocephalus (excess fluid in the area around the brain) and Spina Bifida (birth defect in which the spine fails to develop properly) are preventable and treatable congenital brain disorders only if detected at an early stage. But these remain commonest birth defects in India as the diseases are not being adequately addressed in health policies. ARCHANA JYOTI brings a report ahead of the World Hydrocephalus and Spina Bfida Day being observed on October 25

Hydrocephalus

It is perhaps the most common brain congenital disease which is prevalent all over the world. The incidence is somewhat 80-330/100000 live births. In India, incidence is around 110-120/100000. It means there could be a large burden of this

condition with unknown actual figures, says Dr Daljit Singh, HoD of Neurosurgery Department, GB Pant Hospital, Delhi. He said that to create awareness about Hydrocephalus and Spina Bfida they are organizing a meet at GB Pant Hospital on October 17 with its patients and caregivers.

 What is Hydrocephalus?

It is a disease condition where the water content of the brain increases. It results in raised intracranial pressure, In small kids head enlarges in size more than the normal values.

It results due to an imbalance of water in the brain(CSF) and its circulation or absorption.

What causes Hydrocephalus?

It can be congenital or acquired. Mostly it is due to infection of the brain such as TB, bacteria, and viruses. In addition brain tumours which can block normal flow of Cerebrospinal fluid(CSF) can also produce hydrocephalus.

Congenital causes include aqueductal stenosi, fourth ventricular outlet obstruction,chairy malformation, and some cystic conditions of the brain.

Head injury, brain hemorrhage can also result in hydrocephalus

What are the symptoms of Hydrocephalus?

Headache, vomiting, and a large head are common features of Hydrocephalus in kids. Sometimes head can be very large so that it may be difficult to support the head on the neck. There can be impaired vision, double vision, gait problems. Small kids also show large veins on their scalp.

There can be impairment of scholastic performance and decline in IQ.

How to diagnose Hydrocephalus?

Mostly the diagnosis is based upon clinical symptoms however ultrasound, CT Scan or  MRI brain can help in confirming hydrocephalus as well as its etiology. These investigations are also used in follow up of the case.

What are types of Hydrocephalus?

Broadly HC are of two types. Communicating and non communicating.Communicating HC implies normal flow of CSF but poor absorption in brain. Whereas non communicating which is also called as obstructive hydrocephalus results due to obstruction of flow of CSF in brain.

Additionally hydrocephalus can be classified as Acute or chronic depending upon the rate at which it forms. Acute hydrocephalus are a more serious condition and can be fatal as well.

Does Hydrocephalus lead to seizures as well?

Some kids can have fits and it may be due to impaired blood circulation of the brain due to high intracranial pressure.

Can there be a role of genes in formation of Hydrocephalus?

Certain gene-like mutations in the L1CAM cause X-linked hydrocephalus with aqueduct stenosis.In such cases it runs in family.

What are the treatment options?

Both medical as well as surgical treatment options are available.Drugs like carbonic anhydrase inhibitor can reduce CSF production.

What are surgical options?

Currently shunt surgery is offered to patients wherein a tube is passed from brain to abdomen(VP Shunt).It drains CSF to abdomen thus decreasing brain pressures.

Secondly, endoscopic treatment can be offered to treat various conditions.  Endoscopic third entriculostomy(ETV) is the most common endoscopic procedure performed.

What are the problems with shunt procedures?

Though it is time tested surgery and can be performed easily and has several merits, it can result in complications.Shunt blockage, revisions and infection can happen in best cases and situations.

Almost all cases will require some revision at some stage of life.Yet it can be a life saving operation.

How is endoscopic procedure rated against VP Shunt?

Endoscopic procedures need single incision, much shorter in time, and have no complications that are related to VP Shunt. ETV however can fail, bleeding and infection after ETV can also occur.It doesnot require any foreign body to be put in body like a vp Shunt , so has inherent advantage.

Can endoscopic procedures be done in India?

India is already in the forefront of endoscopic treatment of hydrocephalus.There are more than 100 centres across India where ETV is performed.

Can Hydrocephalus be associated with some other conditions of the body?

Neural tube defects such as Meningomyelocoel is a very common association of hydrocephalus. Sometimes hydrocephalus can develop after surgery of meningomyelocoel. Arnold chiary malformation, Dandywalker syndrome are some other associations of hydrocephalus.

Are there any National level programs to tackle Hydrocephalus?

The Government has introduced  Rashtriya Bal Swasthya Karyakram (RBSK) aiming at early identification and early intervention for children from birth to 18 years to cover 4 ‘D’s viz. Defects at birth, Deficiencies, Diseases, Development delays including disability.

The services aim to cover children of 0 -6 years of age in rural areas and urban slums in addition to children enrolled in classes I to XII in Government and Government aided Schools. It is expected that these services will reach about 27 crores children in a phased manner. Though it covers neural tube defects, it will certainly benefit early screening of hydrocephalus.

Can hydrocephalus be diagnosed in a fetus?

Pregnant females shall get ultrasound of the baby which can hint at occurrence of neural tube defects and hydrocephalus during intrauterine life of the baby.

There are tell-tale signs which can diagnose these conditions fairly accurately.

Can these conditions be treated intrauterine period before birth?

Foetal neurosurgery is a step forward in this direction. Surgery can be performed while baby is still in womb and is put back in uterus after repair.Such children have potential to grow better than those who are operated after delivery

What is the magnitude of Hydrocephalus?

According to the National Institute of Health, hydrocephalus affects one in every 500 live births, making it more common than Down’s syndrome or Deafness. It is the leading cause of brain surgery for children in the United States.

According to some estimates, hydrocephalus may be the most prevalent neurological condition known to medical science, but exact numbers are not known  since no one is keeping data on adults with hydrocephalus.  Estimates put the number of individuals (children and adults) with hydrocephalus between .75 -1.5 million.

Spina Bifida

It is a congenital defect of the spine in which a part of the spinal cord and its covering called meninges is exposed to the outside without skin, muscle or bone cover. It can be very mild, which is called spina bifida occulta in which there is a small gap in the bone but the skin covering is intact

and patient is asymptomatic, picked up incidentally on a chance X-ray, says

Dr Rajendra Prasad, Sr. Consultant Neurosurgeon & Spine Surgeon,  Indraprastha Apollo Hospitals, New Delhi.

In a very severe form it is called myelomeningocele where the spinal cord and its covering bulges out through a defect in a bone, muscles and skin in that part of the spine, usually the lumbar / sacral part of the spine. In most of these cases, the patient presents with weakness of both lower limbs to a varying degree, bowel and bladder incontinence and sometimes also associated with a condition called hydrocephalus where the fluid within the brain cavity also increases resulting in increased head size and may need treatment.

 How can it be avoided?

a)            Use of folic acid tablets during pregnancy: In many patients this condition can be avoided if folic acid tablets are given as early in pregnancy and throughout pregnancy. This condition in urban India is largely reduced because of the use of folic acid by patients, prescribed by their obstetrician / gynecologist.

b)            Early detection through fetal ultrasound: The use of ultrasound in early pregnancyhas helped diagnose early so that the mother is offered a chance to terminate the pregnancy, if she chooses, which

many do.

c)            Avoid certain drugs during pregnancy

(carbamazepine, sodium valproate, Tegretol etc.)

d)            Family History: Some have a family

history of spinal bifida, particularly those who have given birth to spina bifida

children in the past would have higher

incidence of subsequent children having spina bifida.

What is the treatment procedure?

The treatment, once the child is born, is to close the exposed spinal cord and nerves. There is no treatment for the paralyzed lower limbs or lost bladder/bowel function.

These children will require support of the family, care givers, physiotherapy and other supportive measures for the rest of their lives. Many remain wheelchair bound but are able to attend school, their jobs, being of normal intelligence in most of the cases.

So the focus has to be on prevention of Spinal Bifida, both by taking folic acid in

pregnancy and for all pregnant women to undergo fetal ultrasound early in pregnancy at 6-21 weeks as well as subsequently.

“Spina Bfida is preventable by a simple folic acid tablet, which has to be taken by women six to eight weeks before they become pregnant. Since pregnancy is mostly not planned, folic acid supplements ought to be given to all women of reproductive age. To make this possible, awareness is needed, as most women do not even know that they need to be consuming folic acid if they plan on getting pregnant. The government should start a registry for birth defects to understand the actual magnitude of the problem.”

Pediatric surgeon, Dr Santosh Karmakar of Spina Bifida Foundation.

“Babies born with neural tube defects like spina bifida need more than just one surgery to correct the disorder. Since nerves supplying to legs, bladder and rectum get affected, it could lead to locomotor disability, poor bladder and/or bowel control. It requires multidisciplinary intervention which includes pediatrics, orthopedic surgeon and neurologist. Once born with such defects, it is not possible to reverse the damage done.”

Dr Wajid Nazir, Associate Professor, Neurosurgery Department, GB Pant Hospital, Delhi.

“There is currently no known way to prevent or cure hydrocephalus and the only treatment option today requires brain surgery. With early detection and appropriate intervention, the future for many is promising. Untreated hydrocephalus can get worse and lead to brain damage, a loss in mental and physical abilities, and even death. With early diagnosis and timely treatment, though, most children recover successfully.”

Dr Anita Jagetia, Professor, Neurosurgery Department, GB Pant Hospital, Delhi.

“Neural Tube Defects are relatively common problems which actually start happening during the first to fourth week after conception. Hence, a woman planning to have a baby should start taking Folic acid tablets at least four weeks before conception and it should be continued throughout the pregnancy. If a previous pregnancy has been affected by the NTDs then in the next pregnancy the dose of Folic acid should be more. The foods can also be fortified for the women of all age groups. If the woman starts taking Folic acid four months after the conception, then it means she has missed the bus.”

Dr Naveen Gupta, Neonatologist,

Madhukar Rainbow Hospital, Delhi

“While surgery performed after birth can help reduce some of the effects, surgery before birth can prevent or lessen the severity of the fetus’s spinal damage, which worsens over the course of pregnancy.”

Dr Shyaam Bodaliwala, Associate Professor, GB Pant Hospital, Delhi